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Adrenocortical tumor in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Adrenocortical tumour have been described in patients with 21-hydroxylase deficiency. These tumours are usually considered to be ACTH – dependent, as diffuse adrenal cortical hyperplasia is commonly seen. We report adrenal cortical tissue tumours developed in three patients with untreated congenital adrenal hyperplasia due to 21-hydroxylase deficiency. All of them had symptoms of adrenogenital vililizing syndrome. A diagnosis of adrenocortical tumour was established by the symptoms, hormonal profile, ultrasonography, and adrenal CT scan. Two of the tumours were located in the right side, all the patients were performed surgery before hormonal replacement therapy because of evidence of secreated adrenal tumors, and the histological diagnosis indicated an adrenocortical adenoma. After removal of tumours, the size of adrenal gland was monitored by serial ultrasonography, and the congenital adrenal hyperplasia was confirmed by extremely high levels of basal serum testosterone, 17-OHP levels, increasing virilizing syndrome after surgery, diffuse hyperplasia of adrenal gland and identified mutations in CYP21A2 gene.

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Correspondence to Vu Chi Dung.

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Dung, V.C., Thao, B.P., Dat, N.P. et al. Adrenocortical tumor in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Int J Pediatr Endocrinol 2015, P51 (2015). https://doi.org/10.1186/1687-9856-2015-S1-P51

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Keywords

  • Adenoma
  • Testosterone
  • Adrenal Gland
  • Congenital Adrenal Hyperplasia
  • Serum Testosterone